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[Biochemistry](https://durgical.com/en/biochemistry/) · Enzyme

# Ferrochelatase

Ferrochelatase puts iron inside protoporphyrin and finishes heme synthesis in the mitochondrion.

Updated 2026-11-01

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## Key facts

- Protoporphyrin IX + Fe²⁺ → heme + 2 H⁺, on the matrix side of the mitochondrial inner membrane.
- It is a dimer with one [2Fe-2S] cluster per subunit; structure 2QD3 holds the product, heme, in the active site.
- When iron is short it inserts zinc: zinc protoporphyrin forms.
- Lead inhibits it, along with ALA dehydratase.

**Also called**
Heme synthase

**EC number**
[4.98.1.1](https://www.enzyme-database.org/query.php?ec=4.98.1.1)

**Genes**
FECH

**Structure (PDB)**
[2QD3](https://www.rcsb.org/structure/2QD3)

## Regulation

- It depends on iron delivered to the mitochondrion (mitoferrin) and forms a complex with other late-pathway enzymes.

## Inhibitors and toxins

- Lead
- N-methylprotoporphyrins (formed by some drugs in the liver)

## Clinical notes

- Erythropoietic protoporphyria: nearly always a null FECH allele plus a low-expression variant on the other allele.
- Free protoporphyrin builds up in red cells and skin (burning pain in sunlight) and is excreted in bile (gallstones, liver damage).
- High red-cell zinc protoporphyrin: iron deficiency or lead poisoning; high free (metal-free) protoporphyrin: protoporphyria.

## Pathways

- [Heme synthesis](https://durgical.com/en/biochemistry/pathways/heme-synthesis/)

## Related conditions

- [Erythropoietic protoporphyria](https://durgical.com/en/conditions/erythropoietic-protoporphyria/)
- [Lead poisoning](https://durgical.com/en/conditions/lead-poisoning/)
- [Iron deficiency anaemia](https://durgical.com/en/conditions/iron-deficiency-anaemia/)

## Related

- [Protoporphyrin IX](https://durgical.com/en/biochemistry/molecules/protoporphyrin-ix/)
- [Heme](https://durgical.com/en/biochemistry/molecules/heme/)
- [Uroporphyrinogen decarboxylase](https://durgical.com/en/biochemistry/enzymes/uroporphyrinogen-decarboxylase/)
- [Iron metabolism](https://durgical.com/en/biochemistry/topics/iron-metabolism/)
- [ALA dehydratase](https://durgical.com/en/biochemistry/enzymes/ala-dehydratase/)

## Test yourself

Think of the answer, then tap the question to see it.

## What does ferrochelatase do, and where?

It inserts Fe²⁺ into protoporphyrin IX to form heme, in the mitochondrion.

## What forms when ferrochelatase has no iron available?

Zinc protoporphyrin: the enzyme inserts zinc instead of iron.

## Which disease does ferrochelatase deficiency cause?

Erythropoietic protoporphyria, with burning skin pain minutes after sun exposure.

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Educational information, not medical advice. For any symptom, see a health professional.

Sources: [FECH: Ferrochelatase, mitochondrial (human)](https://www.uniprot.org/uniprotkb/P22830/entry) (UniProt, 2026); [FECH gene: MedlinePlus Genetics](https://medlineplus.gov/genetics/gene/fech/) (MedlinePlus, US National Library of Medicine, 2026); [2QD3: Wild type human ferrochelatase crystallized with ammonium sulfate](https://www.rcsb.org/structure/2QD3) (RCSB Protein Data Bank, 2026); [Porphyrins blood test: MedlinePlus Medical Encyclopedia](https://medlineplus.gov/ency/article/003372.htm) (MedlinePlus, US National Library of Medicine, 2026).

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Representative model, for educational purposes only.

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Source: https://durgical.com/en/biochemistry/enzymes/ferrochelatase/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/biochemistry/enzymes/ferrochelatase/